Episode 52: Down the Rabbit Hole: Will I Finally Get a Diagnosis for My Breathing Difficulties?

Worshipping air again, tonight. Holy, sacred, life-bestowing air. Never take it for granted. Yes, I've written on this very topic with some of the very same evidence before, but lack of air tends to become an all-pervasive problem. Air, sports fans, is kind of important.

Photo by Lisa Sheirer
Used with permission

Today I had a horrendous deep, barking coughing fit after I laughed real hard at something Honey said, and I've been coughing and barking since.

I'm wondering now whether this might be the clue that finally makes my pulmonologist believe me when I say how quickly I run out of breath when merely taking a walk or walking up a flight of stairs.

As I've said again and again, this is the most frustrating part of being a patient. My pulmonologist doesn't believe me when I tell him about these symptoms.

Why do I think this?

First, he ordered a six-minute walk test. As the two of us walked, I told the technician that my oxygen saturation would probably be just fine when we stopped at the end of six minutes, but if we waited a minute or two, it would drop. I knew this based on my own observations about the effect of exercise on my ability to breathe.

"That's not how the test works," the tech told me. She measured my saturation just after the six minutes, and it registered 100 percent, and that was the end of it.  After that I sat down and clipped on my own oximeter, and within a couple of minutes, my saturation had dropped to 82 percent. But no one cared about that.

That's not how the test works.

Well, guess what? That's how my lungs DON'T work, and that's why I'm in that office spending medical dollars so I might have some kind of a chance for a life if this thing can be treated.

Fast forward to next visit. I again tell the doc about these symptoms.  Sports fans, these are the very first symptoms that started my recent fall down the rabbit hole of today's health care system. They became debilitating during a trip to Europe during Christmas 2012-New Year's 2013.  I had absolutely zero stamina on that trip for stairs, for walking, for doing anything at all.

It's now a full two years later, and while I've been diagnosed with quite a few things since them--cardiomyopathy, sleep apnea, idiopathic hypersomnolence, kidney disease, and degenerative brain/muscular disease--I think that's it--no one has yet explained this lack of oxygen on the slightest exertion. This isn't just fatigue, which would be expected given all my diagnoses.  This is a very specific reaction to exercise or, to be more accurate, exertion, since even brushing my hair can wear me out.

The first time it happened was well over a decade ago, when my sister and I tackled Mt. Baldy, a mountain in California near our aunt's retirement home.  We took a ski lift up most of the way and were supposed to then take an easy trek across the ridge to the apex at 10,000 feet. We had started at sea level, since we were close to the Pacific Ocean at the base of the mountain, so that's a pretty fast elevation change.

My sister is seven years older than I, and she was trucking along, but my body stopped itself flat and told me, "You ain't taking another step, Girlfriend."  I had to stop numerous times, and starting up again was pure hell.  I finally made it to the apex, but I knew what I'd experienced made no sense, given my then-young age and seemingly good health.

The problem now is that it's happening without shooting up to 10,000 feet in an hour or two.  I also have a history that includes coughing up blood resulting in a bronchitis diagnosis, a bout of pneumonia, and a diagnosis of reactive airway disease a few years ago. And, as I've said a thousand times, there's that damned polycythemia that still shows up in all my lab tests, and the most basic cause for that is a lack of oxygen in the system.

So, back to my trusty medical librarian skills--maybe this severe cough after laughing,which has been happening to me for some time now, will indicate the cause of the problem.

And I think, perhaps, I've found it:  exercise-induced bronchoconstriction. In other words, after exerting oneself, a patient with this problem has trouble breathing because the bronchial tubes, the same culprits in bronchitis, narrow to a potentially dangerous degree. The problem is common in asthma sufferers, but it can also occur in people without any history of asthma.

And laughter, it turns out, affects those bronchii the same way--it is considered a trigger as strong as exercise.

For more on this condition, read about it in this 2011 article from American Family Physician.

I just pray that when I see Dr. P, as I'll call him, this coming Wednesday, this new observation will make him take me seriously.  But I think I may have already told him about this symptom, as I told you, Dear Viewer, in an earlier blog.  I had a major coughing fit while watching a silly scene in an Ace Ventura flick, and that ended with my coughing up about a cup of phlegm. Dr. P. didn't seem to care about that, either. 

During my last appointment, I told Dr. P what had happened with the six-minute test, so he told me to get up and follow him.  He then had me walk up a short flight of stairs in his office building and clipped an oximeter on my finger. His tech again took the measurement and, as I could have told them, of course--no decrease in oxygen saturation.

Well, no duh!  The office building was as cold as ice, and I"ve noticed that heat makes the problem much, much worse. Not only that, I'd just gone to the office after a night on my CPAP--the appointment was first thing in the morning. My symptoms aren't constant, but they are frequent.  And they get worse as the day goes on. The more I do, the worse they get.  It's a cumulative thing.

I tried to explain that to Dr. P, who told me, "If it doesn't happen here in the office, it doesn't happen at all."

I understand doctors need objective evidence. But if a patient consistently complains about a symptom, doing one or two tests that reveal nothing should not be the end of it.  In reading about this condition, I learned that recommended tests include spirometry with and without exertion.  I've had it without exertion twice, but never have I been given that lung test with or after exertion.

And, one of the characteristics of exercise-induced bronchoconstriction is that it happens during or after exertion. So why does the protocol for a six-minute walking test have the oxygen saturation taken ONLY immediately after the end of the six minutes?  What about those of us whose oxygen depletes shortly after that?

"Typical symptoms of EIB include wheezing, shortness of breath, dyspnea, cough, or chest tightness during or after exercise. These symptoms usually occur during strenuous exercise and peak about five to 10 minutes after exercise," state Krafczyk and Asplund in the aforementioned article.

So why would a six-minute walking test NOT check for lack of oxygen for several minutes after the walking ends?

Honestly, I don't frickin' understand what a person has to do to get adequate diagnosis and treatment in this Rabbit Hole known as the U.S. Health Care System.

The cardiologist overseeing my last cardiac stress test told me my heart had performed just fine, "but something is going on with your oxygen." By the end of the test, I was hanging on for dear life; my legs felt as if they were going to give out from under me.

Legs can't run without air.

But nothing about my oxygen, or lack thereof, appeared on the doctor's report of the stress test because, of course, the test was all about my heart function. Well, hello, the two are utterly connected, so why is this?

Even my new physician, whom I'd had high hopes for, told me when I expressed this problem--and that fact that my own oximeter, which my ENT had told me to get when my oxygen was low in his office, drops into the 80th percentile on mild exertion:  "Maybe your oximeter is broken."

I want to scream.  I want to fricking scream.

Maybe there's nothing the doctors can do for my breathing problem, but the frustration of their not taking me seriously and not investigating the problem properly so they and I can know why this is happening to me is driving me absolutely crazy.

I had almost made up my mind that I wouldn't even bother mentioning this problem again this time. It's so discouraging to be made to feel as if I'm making the shit up. But now with this horrendous coughing, I have something else to bring to him. And he'd better take it seriously this time, or I might have to do something desperate. Like write an article and send it to the local paper about the frustration of my quest and the lack of care I'm getting. Don't want to do that. Just want my doctor to do what he's supposed to do.

Remember how excited I was when I was going to see the neuromuscular specialist at Hopkins, who I'd hoped would take my neuro symptoms seriously and give me a DNA test for myotonic dystrophy?  Remember how utterly disinterested and cold she was to me that day, and how disappointing that encounter ended up?

I fear that will happen again this Wednesday.  Going to the doctor shouldn't have to feel like you're about to enter into a battle, but that's exactly how it feels.

"Patients with atypical exertional complaints require careful clinical and physiologic evaluation," says American Journal of Respiratory and Critical Care Medicine authors McFadden and Zawadski.

So why haven't my complaints been carefully evaluated?

A couple of aspects of EID that don't exactly match my situation are that it normally occurs with strenuous exercise, and I haven't been able to do strenuous exercise for a long time. It also occurs tends to occur with "physically active" persons, notably athletes, and I haven't been physically active, much less an athlete, in quite a long time. I've had to give up hiking and bicycle riding. Hell, I've had to give up walking. I've had to give up evenings out with my friends. I've had to give up sex, on a far too frequent basis. I've given up showering and grooming most days. I've given up cooking for myself most days. Every frickin' thing I do exhausts me and leaves me out of breath.

Have I used the word "frickin'" enough times yet? Hey, I'm mad. I'm trying to keep it clean, though.

Certainly I was being physically active when climbing Mt. Baldy those 15 or so years ago. And I first noticed the severity of my symptoms while forced to be physically active during my trip to Europe in 2012, since I had to walk a lot and use stairs in buildings with no elevators. Now, my problems occur with the mildest of exertions, but perhaps that's because the condition has progressed (or regressed) to this debilitating state.

Another problem with the diagnosis is that cold air is known to induce an attack. I, on the other hand, find that hot weather brings on my breathing difficulties much more than cold weather does; however, I do not spend much time in cold weather and certainly don't exercise during cold weather, so I can't really say whether this would affect my breathing or not. The brief trip up the short flight of stairs in the air-conditioning at Dr. P's office wouldn't have induced anything because of the brevity of the test--it was a silly, non-test in my view.

Yes, I'd said that I get out of breath when climbing stairs, but I was talking about the steep, long flight of stairs in the Victorian-era building in which I'd worked at the time. The little flight of wide steps Dr. P tested me on was no comparison.

Krafczyk and Asplund also note that "Self-reported symptoms have been shown to be poor predictors of EIB because other conditions, such as vocal cord dysfunction, can cause similar symptoms. Therefore, symptoms alone should not be used to diagnose EIB."

Okay. I buy that. So give me the right damned test to figure out what the hell is going on--especially since the symptoms can be treated. I would dearly like to do a few things without having to give up in exhaustion because I can't breathe. I'm tired of waiting around for the doctors to believe me enough to give me the right tests.

Let's hope this time, this Wednesday's appointment, things go a little better, and answers are forthcoming. That's all I want. Frickin' answers. What else are my medical dollars paying for? It's like a bad student's being passed from grade to grade. I'm a patient being passed from appointment to appointment. Problem student, problem patient--What's the strategy? Give up on them! Teachers and doctors who do that don't deserve the salaries they draw, in my humble opinion.

I don't want to have to dread my doctor's appointment, but that's exactly what I feel. Dread. And yet I want those answers, so go I will, and do battle I will, if necessary. My quality of life, and my very life, depend on it.

Meanwhile, we just had a snowstorm, and it's bitter cold out. Maybe I'll do a little six-minute walk tomorrow and record the results with my oximeter, taking a photo of those results. Sounds like a good idea to me. But will Dr. P buy it, even then? Most likely he'll just think it's another example of my "obsession" with my health.

Damn straight, I'm obsessed. Anyone would be, if he or she could not breathe properly and could not do much of anything, anywhere, any time. It's miserable.

And I'm damned sick of it all--being sick and exhausted and out of breath and stuck in the U.S. health care system of today, the Rabbit Hole where nothing makes sense. The Rabbit Hole I find myself in today. The Rabbit Hole whose passageways are constricting, tightening, and burying me alive.

___________________

REFERENCES

Krafczyk, Michael A., and Chad A. Asplund. 2011. "Exercise-Induced Bronchoconstriction: Diagnosis and Management." American Family Physician 84(4):427-434.

Parsons, J.P., et al. 2012. "An Official American Thoracic Society Clinical Practice Guideline: Exercise-induced Bronchoconstriction." American Thoracic Society Documents: 1016-1027.

Episode 51: Nature Notes, OR Close Encounters of the Critter Kind

Before his death, biologist Ken Hogsdon published a column in our local newspaper titled "Nature Notes."  I was told when I moved here not to bother subscribing to the paper because it was just a rag for the Good Old Boy Network that ran things around here in those days--and still does, for the most part, which is one reason our biggest "city" was recently named the 6th poorest in the nation.

However, the paper was worth it back in those days because of Hogsdon's charming "Notes." My title is in homage to this man who told stories of his daily, mostly backyard encounters with the region's wildlife.

In that vein, and in an effort to get away from depressing stories about my health, or lack thereof, I am now sharing my own "Nature Notes." Actually, a couple of earlier episodes also shared some "Nature Notes":  Episode 10: Happy Halloween With Some Creepy Plants and Critters, most notably.  (Depending on copyright, I hope to add some of Hodgson's own columns to the show, since I can't find anything about him or written by him on the Internet; his death predated the Web.)

The Cottage

I live in a "cottage," as I like to call it, on the edge of a hardwood forest in Appalachia. My small plot--a mere .6 acres--has several microenvironments: first, the aforesaid forest filled with American basswood, sugar maple, and black oak with a smattering of other species including shagbark hickory.

Beneath the trees grow magical species including Jack-in-the-Pulpit, May apple, trillium, several types of ferns, mosses, and more.

A second environment is what I refer to as my "meadow"--a strip of land along the edge of the forest behind the cottage that is the sunniest location in my yard. My place lies cradled in the side of Big Savage Mountain, which looms to the west and, therefore, blocks most sunlight by 4 p.m. or so even in the summer. This little strip of land holds onto the daylight longer than any other. In the meadow are several types of goldenrod, asters, mugwort, wild phlox, dame's rocket, asters, and more.

Finally, my yard proper makes up the third microenvironment. Actually, my yard isn't so proper: I am slowly allowing low-growing "weeds" (I prefer to call them "wonders") to take over the grass in the hope that someday my lawn will no longer need mowing. Aren't the heart-shaped leaves and happy purple faces of violets preferable to a bunch of straight, boring blades of grass?

Many of my wildlife encounters have taken place on my property, while others occurred in the surrounding beautiful, mountainous terrain.

Here are a few stories to begin this "series."

The Earth Moved

Outside my back door is a concrete slab I hope to someday make into a flagstone patio. I often leave items such as flowerpots needing planting back there. One summer evening I came home with some new flowers, so I chose a fairly large terracotta pot filled with dry soil as their home. I carried in the pot and put it in the sink, turned on the faucet, and walked away since I knew the hard soil would need a good soaking

From across the room, however, I glanced over at the flowerpot and, to my shock and momentary horror, the soil began to GROW.  The dirt rose up in a large lump, and my heart stopped as my brain tried to process what the heck was going on. The soil began to shape itself into a sloped head with eyes blinking.

And then I realized what I was seeing--a toad, who had made his home in this warm little pot of earth, had experienced his own rude awakening, and he also wanted to know what was going on! He looked like the biggest toad on earth, all covered in soil, as I sprang across the room and gently covered the flowerpot with a saucer and took my new friend back outside--whereupon he quickly vacated what must have seemed like the ideal place to hang out. At night, my porch light would have attracted to his lair all the tasty morsels he could eat!

Had I known of this squatter, I would have happily allowed him the real estate rather than ungraciously evicting him in favor of a mere flower.

Tiny Ring-Neck Snake

Other objects end up on that concrete slab, and one day I picked up a piece of wood I'd placed there for some reason, and beneath it was the tiniest snake I'd ever seen.  I hadn't even known snakes came that small!

This one was about two inches long and very slender, all black, other than a bright yellow ring where its neck might be said to be. This was a hatchling of a fairly common type of snake in the area, the Northern Ringneck Snake (Diadophis punctatus), though I didn't know that at the time.

No, this isn't my hand--
Photo from the Savannah River Ecology Laboratory

For all its evils, the Internet sure is a wonderful innovation. Anything that piques my curiosity can easily be answered with a few choice search words. In this case, "ring" and "neck" and "snake." Can't get easier than that!

Green Greetings

Admittedly, my threshold for thrilling surprises may be low, but I'll take these little green surprises--each of them found on rocky ledges--over a surprise mink coat any day.

The first happened many moons ago when my two boys and I began exploring our new environment after moving from Washington, DC, to Appalachia. At the time, J., my older son, was about ten years old and had bought a book on West Virginia at the local bookstore, after which he hounded me to visit all the wild, wonderful places in that closely neighboring state. Well, "hounded" is probably too strong a word--it didn't take much encouragement at all!

That's how we ended up numerous times at the rather amazing Dolly Sods Wilderness, an apparent remnant of the Ice Age when glaciers covered this part of our country. The vegetation is characteristic of much more northern (as in northern Canadian) terrain and includes sphagnum bogs and heaths on its rocky mountaintop--not to mention a bounty of blueberry bushes, as we found one fall when they were bursting with fruit. Son J. was also fascinated with the area's "flag-form trees"--trees with branches growing only on one side due to the intensity of winter winds on the boulder-strewn mountaintop.

Dolly Sods Wilderness at Bear Rocks - Photo from Nature Conservancy

The strange name, according to Wilderness.net, is for the Dalhe family who "used the open grassy fields called 'sods' for grazing sheep in this area." Even stranger are the signs on all the trails warning that the area was a pre-World War II training area--and if a hiker were to find a stray mortar round in the "wilderness," he or she should leave it alone since it could still be live! Talk about cognitive dissonance!

105-mm Howitzer round found at Dolly Sods--
Gee, what a symbol for the masculine nature of war!
Photo from West Virginia Highland Voice

Meanwhile, younger son A., who loved to complain about hiking, nevertheless had a great time jumping from rock to rock in a heart-stopping manner whenever we arrived at outcroppings. After one of his treks to the edge of the Dolly Sods plateau, he came running back for me, calling out, "Come here! Look what I found!"

So I followed him down a few boulder stairs and looked where he was pointing--and there, curled happily on a miniature cave-like rock ledge, was a Smooth Greensnake (Opheodrys vernalis) in all its emerald glory. Again, I'd had no idea snakes came like this!

Photo of Smooth Greensnake from Maryland's Department of Natural Resources

A couple of decades later (sigh--really?) a similar green encounter greeted me most unexpectedly. This time, I was in a canoe, resting with a beer as my Honey paddled us around the Savage River Dam, a reservoir surrounded by the Savage River State Forest, a setting not unlike, I imagine, the Scottish lochs my Honey's and my ancestors enjoyed.

We floated along a shore bounded by a wall of rock--a wall with plenty of crevices and ledges for other creatures wanting to enjoy the fine day and the lovely body of water--including, in one, a small, brilliantly green frog! 

Tonight I searched Maryland's Department of Natural Resources site to try to figure out what these species might be, which is how I identified the Smooth Greensnake (there's also a Rough Greensnake but it does not live in our neck of the woods, while the smooth one does).

The only bright green frog I could find is the Green Treefrog (Hyla cinera), and its description fits our sighting: Their habitat includes "Swamps, borders of lakes and streams, floating vegetation . . . ." This little guy was indeed on the border of the lake, perched happily on his little balcony where he, no doubt, waited for flying insects to try to pass by.

Photo of Green Treefrog from Maryland Department of Natural Resources

Swimming Salamanders

The frog-occupied rock ledge holds back a finger of land that, on its other side, has a rocky beach easy to "land" on with our canoe.  Last summer, Honey and I pulled up on the beach and walked over to the other side where we sat on the ledge and enjoyed the view.

"Remember that green frog we saw?" I said--we'd seen it on one of our first canoe voyages on the Dam, as we call it--probably some nine years ago, back when our relationship was tenuous and I never imagined I'd be back there with him nearly a decade later.

In fact, he is another reason the local newspaper was worth reading back then--he was the editorial page editor ten years ago; I wrote a letter to the editor; and the rest is History. Actually, so is his job at the paper, because that Good Old Boy Network didn't like the politics in his columns and, in an affront to journalistic integrity everywhere, told him to pack up and leave after fourteen unblemished years of employment. Needless to say, I don't read that rag anymore.

BUT I digress. Back to happier topics:

I looked over the ledge and, to my surprise, saw a populous little world I'd had no idea of--dozens of salamanders swimming around, and even a few that had ventured onto the hard, rocky ledge where we sat! I hadn't noticed the ones on land because they were so well camouflaged. The place seemed a harsh landing spot for salamanders, as it was covered with nothing but red dirt and small pebbles, but perhaps they, like the green frog, also pulled up into the shaded rock crevices below when they needed a rest.

No hope in identifying what they might have been from memory, as many species of salamanders live in our region, and these were brown and nondescript, as many of them are. They may well have been the adult version of our newt Ethel, an Eastern Red-Spotted Newt (Notophthalmus viridescens) who is in her terrestrial juvenile stage, known as "eft."

Ethel, our Eastern Red-Spotted Newt Eft, in her Florida room,
actually an intact old French's mustard jar we found in the woods

More Ethel

I found Ethel, and her now-deceased mate Fred, in my yard after a spring rain, like orange jewels among the brown leaf litter. Actually, efts are neither male nor female; newts do not become sexual beings until their adult, mostly aquatic (and mostly brown) stage.

Next summer, I'm going back to that ledge with a field guide in tow, and maybe I'll be able to figure out what species lives so thickly in that little part of the lake.

One thing I do know--they weren't hellbenders! Hellbenders are endangered and huge (for a salamander); in fact, they're known as giant salamanders, and the few that are left live in Appalachian streams near us. Now, that would be a sighting, and I haven't given up on seeing one yet.

Adult Eastern hellbender, photo from Maryland Department of Natural Resources

In the meantime, I'll just enjoy a giant burrito at Hellbender's Cafe in Davis, West Virginia, another "find" when the kids and I used to explore the state! Now, THOSE are monsters!

GREAT big burritos at Hellbender's in Davis, West Virginia

More Nature Notes to come!

Episode 50: Down the Rabbit Hole and In the Third Stage of Grief?

Kubler-Ross's five stages of grief upon learning of one's imminent demise might also be relevant in acceptance of severe chronic disease.

I've gone through the first two, and will no doubt cycle back now and then:

1) Denial - That stage didn't last long for me. It was hard to argue with my symptoms, hard to deny that my body was failing me.

2) Anger - This, on the other hand, is still pretty fresh. I'm not so angry at the gods--I've been blessed in so many ways in my life, and why should I be immune to pain or "injustice," if that's what illness is, when others on this planet suffer so greatly?

I have often, however, been angry at the health care system and at individual health care providers for a variety of valid reasons, many of which have been described here on The Mary Dell Show.

And although I get upset when I start thinking about how a fragmented system has betrayed me, and how individual physicians and practices have made me feel marginalized, I'm moving beyond anger now.

Why get angry? That's just more energy to expend, when I have so little energy anymore at all.

And so I'm moving into that third stage, bargaining.

Episode 49: No DNA Results Yet, But Another Delightful Hallmark of Myotonic Dystrophy (Fecal Incontinence) Warms my Christmas

Merry Christmas!  And Happy Day, if you don't happen to celebrate Christmas.

My Christmas was greeted with yet another symptom of myotonic dystrophy, the particular gem in a group of symptoms that can, in fact, first lead to a myotonic dystrophy diagnosis for many patients: "Digestive symptoms may be the first sign of dystrophic disease and may precede the musculo-skeletal features," state Bellini, et al. (2003) (full reference at end of episode).

In the past, I've suffered this especially special symptom, among other, nearly daily digestive symptoms, to quite debilitating measures.  And then it mysteriously disappeared, for the most part, though occasionally revisiting to remind me how little control any of us has when our bodies lose theirs.

Yes, happy, happy, joy, joy!

This Christmas gifted me with another fun day of fecal incontinence.

It happened just as we'd finished opening presents around the tree with Honey's two kids--his third is, sadly, suffering something along the lines of agoraphobia--fear of leaving the house due to panic attacks, and stayed at her mother's house for the day. Meanwhile, my two, both adults, are 1.5 hours away, one celebrating the holiday with his girlfriend and the other with their Dad, who has a big family filled with my boys' aunts, uncles, cousins--for a Christmas filled with masses of good food and cheer.

Here at Honey's, thankfully most of the kids had cleared the room when, out of the blue, the contents of my guts oozed out of my body into the seat of my Christmas jammies from several years back, my favorites, covered with tiny Miniature Schnauzers but now with something extra, and most unwelcome. I had to ask Honey's son to leave the room, and the look I gave Honey told him all he needed to know.

He'd lived through the times with me when this horrible condition had plagued me for months (several years ago), culminating in a particularly hideous and voluminous "lava" flow while shopping, and my subsequent refuge in a fortunately unoccupied women's bathroom where I had to wash out my jeans and wear them wet back to the car and home, thanking the gods for not exposing myself or anyone else to the humiliation of witnessing the incident. And having a good cry before going anywhere, cold and wet and disgusted.

Fortunately, today, the Christmas jammies could be discarded, and nothing had seeped into the furniture.

I tell this story, hopefully with a little humor, to let others out there with fecal incontinence know that they are not alone.  It's not a topic one discusses in polite company, so, what the hell, we are not polite here at the The Mary Dell Show. We tell it like it is, warts and all. Seeping anuses and all.

After getting myself squared away, I retreated to bed where my hypersomnolence kept me obliviated from the rest of Christmas Day. I couldn't even get up to make my signature turkey gravy, much to everyone's disappointment, though it's now 4 a.m. and I have the juices with which to make gravy for leftovers. Yikes, kind of a weird thing to talk about when I've just described how my body just expelled its own juicy brown liquid!

Turkey gravy, anyone?

Unfortunately, I had another little, NOT catastrophic, episode of fecal incontinence tonight while wearing my NEW Christmas jammies, so they are being washed and bleached as we speak. And this second prompted my need to discuss this worst of symptoms with you, Dear Viewer, my non-judgmental friend.

My DNA test for myotonic dystrophy has been completed, but I still don't know the result. I did, however, receive the bill: over $6,000.  I'm responsible for it because Dr. RQ at Johns Hopkins Neuromuscular Clinic did not believe such a test was warranted, but my local neurologist asked if I'd be willing to pay for the test (about $1,000, he'd told me). He ended up ordering a battery of myotonia tests, which is why it was so much more. I do have a form to fill out that will lessen the bill because my federal disability benefits were recently approved, and those on Medicaid can have the amount reduced.

Perhaps I won't know the results of the DNA test until the bill is paid, or perhaps the company sent the results to Dr. A., my neurologist. At this point, I'm kind of afraid to know. I hate the thought of spending hundreds, possibly thousands of dollars I don't have on a negative test, but I still say it's worth it to know, yay or nay. If I don't have myotonic dystrophy, that's good news, indeed. But if I do have this genetic condition--and I have nearly every sign and symptom, including these digestive ones, of the disease--members of my family, especially those of a younger generation, have a right to know they have these defective genes, if for no other reason than saving them the trouble and expense of a long medical investigation into mysterious symptoms.

And I, in fact, have symptoms throughout my gastrointestinal symptom from my mouth (enlarged tongue) all the way to, well, you know where.

"Myotonic dystrophy (MD) is characterized by myotonic phenomena  and progressive muscle weakness. Involvement of the gastrointestinal tract is frequent and may occur at any level," begins the abstract for an article on these conditions by Bellini, et al, 2006. Our digestive tract involves a series of "smooth" muscles, and these muscles can become weakened in a number of neuromuscular diseases.

As for myotonic dystrophy, "In the upper digestive tract, dysphagia, heartburn, regurgitation and dyspepsia are the most common complaints, while in the lower tract, abdominal pain, bloating and changes in bowel habits are often reported."

Okay:
Dysphagia (trouble swallowing):  Check, but mild and sporadic;
Heartburn:  Check.  Very bad, actually.  Diagnosed by ENT and with a hiatal hernia, and prescribed meds.
Regurgitation:  Check.  I've told doctors numerous times that I fairly frequently cough up stomach contents, but no one, apparently, cares about that.
Dyspepsia:  Check.  Upset stomach, basically.  Very frequent.
Adominal Pain:  Check.  Frequent.
Bloating:  Check.  Frequent.  Often with very forceful belching.
Changes in bowel habits:  Well, that's the topic of the day, isn't it?

I went through months, perhaps as much as a year, with fecal incontinence as well as daily watery diarrhea--I don't think I had a normal bowel movement that entire time. No cause was found, and eventually it sort of resolved, though occasional bouts, as I said earlier, returned.

Over the past year, I'd say I've been constipated more frequently than I've had to deal with diarrhea, but I've had episodes of both.

I'm always aware of my digestive system, on one end or the other, and I don't want to even think about my digestive system.

But when yours isn't working, you will think about it. You have to. Or you may find yourself very embarrassed one day. Of course, thinking about it won't guarantee you won't have an embarrassing moment, such as mine this morning when I had to ask my step-son to leave the room. I am just so fortunate to have an understanding, loving partner who immediately knew what the problem was and helped get me into the bathroom.

All in all, it's been a good Christmas. Parts of it have been sad, including a weird rift with one of my sons that I don't even understand and that hurts pretty bad. I'm trying not to think about that; I'm trying to stay happy for the season.

I'd hoped to bake cookies all day Christmas; instead I was in bed, in that nether-nether world where I find myself more often than in waking reality these days.

And then there's the fecal incontinence thing. At least I was home, in the bosom of part of my family, and not out in some mall, when the flood gates opened.

Let's hope for a Happy New Year.

A new beginning, on my son's birthday, the first baby born in Washington, DC, in 1983. I love you, little one now grown, as well as your big brother. The two of you have brought more blessings than I can count into my life, and you are the very best of my accomplishments. I wish I weren't falling apart, and I'm sorry you have to deal with a falling-apart mom. This is not what I would have wished for you two or for me.

All we can do is try to deal with our falling apart with a little humor. Even the gross stuff.  Which is what I tried to do today, despite never finding scatalogical humor very funny.

Har har har on Ho Ho Ho Day.

Here's a group that has been fighting back against often debilitating Irritable Bowel Disease with humor for years--and I say, Cheers and More Power To You, Great Bowel Movement!

Let's hope this New Year gives us a Depends-free twelve months--God bless all of you who have to resort to these measures; I imagine I'll be joining you soon.

Being chronically ill ain't fun, sports fans.

___________________________

Bellini, Massimo, et al. "Gastrointestinal Manifestations in Myotonic Dystrophy." World Journal of Gastroenterology 12:12 (1821-8), 2006.

Degraeuwe, J, et al. "Faecal Incontinence Due to Atrophy of the Anal Sphincter in Myotonic Dystrophy." Acta Gastrtoenterology Belgium 74:1 (88-90), 2011.

Episode 48: I Am Not Alone Down the Rabbit Hole: THE ATLANTIC's "Doctors Tell All--And It's Bad"

The Atlantic recently published an essay by a patient (also the daughter of a patient) whose experiences in today's health care system are as corrosive as those I've been pondering in The Mary Dell Show since Day One.

In fact, the author, Meghan O'Rourke, uses the same metaphor that I have for years for that health care system: "In the hospital, I always felt like Alice at the Mad Hatter's tea party: I had woken up in a world that seemed utterly logical to its inhabitants, but quite mad to me." Those words are nearly verbatim from one of my Down the Rabbit Hole episodes published long ago.

Original illustration of the Mad Hatter's Tea Party
in Alice's Adventures in Wonderland

O'Rourke also compares the amount of blood she gives to the stores of a vampire, as I did after having phlebotomies (blood letting) as treatment for my polycythemia. And how many times have I compared "essential or idiopathic this or that" to doctor-speak for physicians' not knowing what the hell is going on?  As, in fact, she does as well.

O'Rourke invokes the same phrase I often complain about when describing life Down the Rabbit Hole of today's medical system, one dispensed by that System like sugar pills:  "patient-centered care." That, I'm afraid, is a stark euphemism for "the patient comes last."

And O'Rourke agrees:  "For a system that invokes 'patient-centered care' as a mantra, modern medicine is startingly inattentive--at times actively indifferent--to patients' needs."

Well, isn't that exactly what I've been saying all along here?  In fact, the article started to get to me--it was as if O'Rourke had pulled phrases right out of my own record, though I'm not accusing her of doing that. It's simply, I suppose, that another literate patient has been given the run-around I have, and the same allusions and phrases occurred to her.

Big surprise.  All patients are being given that run-around.  Nearly every patient in today's health care system will fall down that Rabbit Hole.

Episode 47: Hypersomnolence, OR Sleeping Too Damned Much, OR I Want My Life Back

I've mentioned hypersomnolence on the show many times, but what exactly does it mean?  How does hypersomnolence affect one's day-to-day life?

A typical day.

Of course, "hyper" means too much of something, and "somnolence" means sleepiness. And I have been dealing with this strange and debilitating condition for the past six-plus years. The official diagnosis is idiopathic hypersomnolence; "idiopathic" simply is medspeak for "We don't know why the hell it's happening." More frequently than not, it seems, idiopathies end up being caused by brain pathologies, and recent studies on this condition point toward the same.

Another term used is primary hypersomnolence; this means the problem is due to its own cute self and not something else going on in the body.

Another typical day.

Hypersomnolence can also occur due to other health conditions, such as cancer, in which case it's known as secondary hypersomnolence. Whatever is happening to me is slow-moving, and most cancers aren't, so I'm not overly concerned about that. As any faithful viewers know, I suspect I may have myotonic dystrophy and am waiting for my DNA results as we speak. This is a genetic neuromuscular condition, and one of its hallmarks is--you guessed it, sports fans, hypersomnolence. But whether or not I have myotonic dystrophy, there's no doubt (and I have scientific evidence, as described below) that I have, indeed, been blessed with hypersomnolence.  Lucky me.

And, yes, yet another typical day.

I've always tended toward sleepiness, particularly having trouble waking and getting out of bed in the mornings and, in recent years, the afternoons and even some evenings. Yet I managed to get going all through school and most of my work years, kicking and screaming all the way. I used to sleep in until 1 or 2 on the weekends and would feel a little guilty for it--but that was nothing compared to what I've experienced since 2006 or 7.

Episode 46: With Accolades and Self-Flaggelating Gratitude for the Most Eminent Human Doctor A, OR Thanks, Good Doctor, for the DNA Test

The title just about says it all. But, for the long version, read on:

This past Wednesday I saw Dr. A for my routine neurology checkup--this one, of course, the first after having met my nemesis in a bizarrely unhelpful neurologist at Johns Hopkins Neuromuscular Clinic, which actually made this local appointment slightly less than routine.

Yet I felt defeated before going in. What would be the point of trying to convince Dr. A that Dr. Red Queen (RQ) of Hopkins had not properly examined me or considered my medical history when she'd come up with her unequivocal veto of any possibility that I might actually have myotonic dystrophy?

If the woman had spoken to me with the slightest curiosity or compassion, I might have more easily accepted her assessment. Unfortunately, I maintain to this day that she subjected me to quite the opposite.

But this is not meant to rehash a story already told in a previous episode.

The issue at hand today had everything and nothing to do with Dr. RQ.

Trying to convince one doctor that another has treated you badly is akin to having to convince someone of your sanity.  Try it sometime.

However, I've have learned through long, hard experience that doctors are not infallible, beginning when I, at twenty years of age, disagreed with my eldest son's pediatrician who told me my firstborn, then about five months old and about whom fellow church members had expressed concern he might have hydrocephalus, was merely a "right-handed baby with a funny-looking head." That's exactly what he said--I realize now in a possibly unfortunate attempt to sound lighthearted and relieve my worry.

But I had reason to worry back then.  My insistence that my baby be seen at Children's Hospital National Medical Center revealed his skull was closing up prematurely and, if it weren't opened by a surgeon, would result in terrible brain damage--a condition called craniosynostosis. Oh, and he wasn't just "right handed"--he'd held his chubby little fist to his chest because his premature birth had resulted in a cerebral hemorrhage. His entire left side was partially paralyzed. And the reasons for all of the problems, I might add, are consistent with pregnancies in women with myotonic dystrophy.)

My point is this:

I don't always agree with a physician tells me, though if one were to mount a study on the many encounters I've had with doctors of many types and specialties one would conclude I've been a respectful and mostly cooperative patient. I'm just not willing to give up my right to think for myself.